Myung Hyun Cho1, Yo Han Ahn2, Seon Hee Lim2, Ji Hyun Kim3, Il-Soo Ha2,4,5, Hae Il Cheong2,4, Hee Gyung Kang6,7,8,9. 1. Department of Pediatrics, Hallym University Sacred Heart Hospital, Anyang, Republic of Korea. 2. Department of Pediatrics, Seoul National University Children's Hospital & College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea. 3. Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Republic of Korea. 4. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea. 5. Kidney Research Institute, Medical Research Center, Seoul National University College of Medicine, Seoul, Republic of Korea. 6. Department of Pediatrics, Seoul National University Children's Hospital & College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea. kanghg@snu.ac.kr. 7. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea. kanghg@snu.ac.kr. 8. Kidney Research Institute, Medical Research Center, Seoul National University College of Medicine, Seoul, Republic of Korea. kanghg@snu.ac.kr. 9. Wide River Institute of Immunology, Seoul National University, Hongcheon, Republic of Korea. kanghg@snu.ac.kr.
Abstract
BACKGROUND: Typical hemolytic uremic syndrome (HUS) causes acute kidney injury (AKI) and serious sequelae of chronic kidney disease (CKD) in some. Hyperuricemia is a common finding in typical HUS that may contribute to kidney damage. We explored whether aggressive management of hyperuricemia with rasburicase could improve outcomes in AKI patients with typical HUS. METHODS: We retrospectively analyzed medical records of children with typical HUS admitted to a tertiary center between 2005 and 2017. We compared clinical outcomes of hospitalization and 1-year post-discharge between those with rasburicase treatment (n = 13) and those without (controls, n = 29). RESULTS: With rasburicase treatment, hyperuricemia corrected more rapidly (median 36 vs. 120 h, p < 0.001), and hospital stays were shorter (median 9 vs. 12 days, p = 0.003) than in the controls. There was no difference in dialysis requirement. At 1-year post-discharge, the proportion of patients with impaired kidney function (estimated glomerular filtration rate < 90 mL/min/1.73 m2) was lower in the rasburicase group (7.7% vs. 41.4%, p = 0.036) than in the controls. Hypertension and proteinuria tended to be more common in the controls than in the rasburicase group. Collectively, long-term renal sequelae of impaired kidney function, proteinuria, or hypertension at a 1-year follow-up was less common in the rasburicase group than in the controls (7.7% vs. 62.1%; p = 0.001). CONCLUSIONS: Children with typical HUS treated with rasburicase had shorter hospital stays and less long-term sequelae at 1-year post-discharge than those who were not treated with rasburicase. These results support the use of rasburicase to prevent CKD in pediatric patients with typical HUS-associated AKI. Graphical Abstract.
BACKGROUND: Typical hemolytic uremic syndrome (HUS) causes acute kidney injury (AKI) and serious sequelae of chronic kidney disease (CKD) in some. Hyperuricemia is a common finding in typical HUS that may contribute to kidney damage. We explored whether aggressive management of hyperuricemia with rasburicase could improve outcomes in AKI patients with typical HUS. METHODS: We retrospectively analyzed medical records of children with typical HUS admitted to a tertiary center between 2005 and 2017. We compared clinical outcomes of hospitalization and 1-year post-discharge between those with rasburicase treatment (n = 13) and those without (controls, n = 29). RESULTS: With rasburicase treatment, hyperuricemia corrected more rapidly (median 36 vs. 120 h, p < 0.001), and hospital stays were shorter (median 9 vs. 12 days, p = 0.003) than in the controls. There was no difference in dialysis requirement. At 1-year post-discharge, the proportion of patients with impaired kidney function (estimated glomerular filtration rate < 90 mL/min/1.73 m2) was lower in the rasburicase group (7.7% vs. 41.4%, p = 0.036) than in the controls. Hypertension and proteinuria tended to be more common in the controls than in the rasburicase group. Collectively, long-term renal sequelae of impaired kidney function, proteinuria, or hypertension at a 1-year follow-up was less common in the rasburicase group than in the controls (7.7% vs. 62.1%; p = 0.001). CONCLUSIONS:Children with typical HUS treated with rasburicase had shorter hospital stays and less long-term sequelae at 1-year post-discharge than those who were not treated with rasburicase. These results support the use of rasburicase to prevent CKD in pediatric patients with typical HUS-associated AKI. Graphical Abstract.